We report on a primary mediastinal large B-cell lymphoma with aberrant expr
ession of P-human chorionic gonadotropin (beta-hCG). The patient, a 33-year
-old man, had cough, dyspnea, fever, superior vena cava syndrome, and a med
iastinal bulky tumor. A biopsy showed that the latter was characterized by
large cells, sclerosis, and compartmentalization. The neoplastic elements e
xpressed CD45, CD20, CD79a and, partially, CD30, whereas they were negative
for CD3, epithelial membrane antigen and cytokeratins. Surprisingly, they
displayed a clear-cut positivity for beta-hCG. The remaining oncofetal mark
ers applied (PLAP and alpha(1)-fetoprotein) were negative. Electron microsc
opy demonstrated the presence of numerous nuclear pockets and the lack of i
ntercellular junctions. DNA analysis by polymerase chain reaction showed cl
onal rearrangement of Ig heavy-chain genes. The patient responded promptly
to the administration of MACOP-B. To the best of our knowledge, this is the
first example of B-cell lymphoma showing positivity for beta-hCG; a simila
r aberrant expression was previously observed only in three Japanese patien
ts with human T-cell lymphotropic virus type I+ adult T-cell lymphoma/leuke
mia. Because primary mediastinal large B-cell lymphoma has in the past been
frequently confused with germ cell tumors, pathologists should be aware of
possible beta-hCG expression by lymphomatous cells to avoid the risk of mi
sdiagnosis.